Susac syndrome - A review of current knowledge and own experience

Otolaryngol Pol. 2023 Jan 31;77(3):20-25. doi: 10.5604/01.3001.0016.2288.

Abstract

Susac syndrome is a rare connective tissue disorder. The pathology affects the small vessels of the brain, retina, and inner ear, and therefore the main symptoms of the disease include encephalopathy, visual disturbances, and sensorineural hearing loss. The aim of this article is to review the current medical knowledge on Susac syndrome and to present our clinical experience regarding this disease entity. In the paper, we are also presenting a case of a 25-year-old patient who was diagnosed with Susac syndrome on the basis of clinical presentation and additional test results. Susac syndrome should be differentiated from multiple sclerosis and other causes of multifocal brain damage as early diagnosis and treatment play a key role in later prognosis.

Keywords: Susac syndrome; autoimmune vasculopathy; encephalopathy; hearing loss; retinal occlusions.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Brain / pathology
  • Brain Diseases* / diagnosis
  • Brain Diseases* / etiology
  • Brain Diseases* / therapy
  • Humans
  • Magnetic Resonance Imaging / adverse effects
  • Retinal Artery Occlusion* / diagnosis
  • Retinal Artery Occlusion* / etiology
  • Retinal Artery Occlusion* / therapy
  • Susac Syndrome* / complications
  • Susac Syndrome* / diagnosis
  • Susac Syndrome* / therapy